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TREX1 Antibody

  • 货号：
    CSB-PA865133ESR2HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA865133ESR2HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) TREX1 Polyclonal antibody
  • Uniprot No.：
    Q9NSU2
  • 基因名：
    TREX1
  • 别名：
    TREX1 antibody; Three-prime repair exonuclease 1 antibody; EC 3.1.11.2 antibody; 3'-5' exonuclease TREX1 antibody; Deoxyribonuclease III antibody; DNase III antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Three-prime repair exonuclease 1 protein (1-270AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Major cellular 3'-to-5' DNA exonuclease which digests single-stranded DNA (ssDNA) and double-stranded DNA (dsDNA) with mismatched 3' termini. Prevents cell-intrinsic initiation of autoimmunity. Acts by metabolizing DNA fragments from endogenous retroelements, including L1, LTR and SINE elements. Unless degraded, these DNA fragments accumulate in the cytosol and activate the IFN-stimulatory DNA (ISD) response and innate immune signaling. Prevents chronic ATM-dependent checkpoint activation, by processing ssDNA polynucleotide species arising from the processing of aberrant DNA replication intermediates. Inefficiently degrades oxidized DNA, such as that generated upon antimicrobial reactive oxygen production or upon absorption of UV light. During GZMA-mediated cell death, contributes to DNA damage in concert with NME1. NME1 nicks one strand of DNA and TREX1 removes bases from the free 3' end to enhance DNA damage and prevent DNA end reannealing and rapid repair.
  • 基因功能参考文献：
    1. data identifies miR-103 regulation of TREX1 as a potent modulator of the tumour microenvironment. PMID: 27886180
    2. Thus, the intracellular level of TREX1 pivotally modulates innate immune induction by HIV-1. Partial HIV-1 genomes are the TREX1 target and are sensed by cGAS. PMID: 29769349
    3. TREX1-deficient neurons also exhibited increased apoptosis and formed three-dimensional cortical organoids of reduced size. PMID: 28803918
    4. Homozygote R114C mutation in TREX1 gene was shown in two siblings, one with chilblains and another with chilblains accompanied with cerebral vasculitis. PMID: 28919362
    5. TREX1 frame-shift mutations induce autoantibodies against mostly non-nuclear antigens, patients carrying these mutations are likely be ANA negative PMID: 28325644
    6. The activation of interferon-induced genes is controlled by the JAK-STAT system; therefore, JAK inhibitors were successfully used in several cases to treat type 1 interferonopathies. Experience with this treatment modality is continuously growing. PMID: 28389709
    7. TheTREX1 and STING, which are opposing regulators of the cytosolic DNA-sensing pathway. PMID: 28475463
    8. the cell-cycle-dependent post-translation modification of TREX1 regulates its interaction with OST. PMID: 28297665
    9. The effect of topical TREX1 knockdown and local interferon production on HIV transmission in human cervicovaginal explants and humanized mice, is reported. PMID: 27184854
    10. data do not support the concept of retroelement-derived cDNA as key triggers of systemic autoimmunity in Trex1-deficient humans and mice PMID: 28835460
    11. Aicardi-Goutieres syndrome protein TREX1 suppresses L1 and maintains genome integrity through exonuclease-independent ORF1p depletion. PMID: 28334850
    12. Analysis of mRNA expression profiles in breast tumors demonstrates that those with lower Trex1 and higher BLM and EXO1 expression levels are associated with poor prognosis PMID: 28279982
    13. showed that patients with SSc or SLE have AAb against EphB2, a protein involved in angiogenesis, and THEX1, a 3'-5' exoribonuclease involved in histone mRNA degradation. PMID: 27617966
    14. The expression of TREX1 was closely related to the cytobiology characteristics of osteosarcoma stem cell. PMID: 27881153
    15. Heterozygous mutations in TREX1 were reported in SLE patients. PMID: 26150267
    16. synonymous variant p.(Tyr177) associated with an increased risk of systemic scleroderma; association strongest in patients with limited cutaneous scleroderma, in particular in those with more severe digital vascular disease PMID: 27574969
    17. This study reviewed that Neurologic Phenotypes Associated with Mutations in TREX1 in patients with Aicardi-Goutieres Syndrome. PMID: 27643693
    18. Aicardie-Goutieres syndrome is described in a patient with a homozygous p.Arg114His mutation in the TREX1 gene. PMID: 28089741
    19. CDK11 was found associated with the TREX/THOC, which recruited this kinase to DNA. Once at the viral genome, CDK11 phosphorylated serines at position 2 in the CTD of RNAPII, which increased levels of cleavage and polyadenylation factors at the HIV 3' end. In its absence, cleavage of viral transcripts was greatly attenuated. PMID: 26567509
    20. Aicardi-Goutieres syndrome 1 is caused by mutations in the three prime repair exonuclease 1 gene (TREX1, MIM 606609). PMID: 25906927
    21. Functional analysis of retinal vasculopathy with cerebral leukodystrophy-associated TREX1 T270 frameshift-mutated fibroblasts showed a prevalent localization of the protein in the cytosol, rather than in the perinuclear region PMID: 25213617
    22. Here we briefly describe the human diseases so-far associated with mutations in TREX1, which include Aicardi-Goutieres syndrome, familial chilblain lupus, systemic lupus erythematosus and retinal vasculopathy with cerebral leukodystrophy PMID: 25731743
    23. Studies indicate that the biosensing strategy is based on the protection of DNA duplex from exonuclease III (Exo III)-mediated digestion by specific binding of estrogen receptor (ER) to its DNA response element. PMID: 23681011
    24. The s observed that the minor allele of SNP rs3135941 in Trex1 is associated with faster HIV-1 disease progression. PMID: 25162766
    25. Unlike previously reported TREX1 mutations, which affect Exo I or Exo II domains, the mutation presented here alters the Exo III domain, suggesting a role of mutations within the catalytic Exo domains in the pathogenesis of familial chilblain lupus PMID: 25517357
    26. Data suggest that the 3'-5' DNA exonuclease TREX1 may contribute to stabilization of poly(ADP-ribose) polymerase-1 (PARP1) levels in the DNA damage response and its activity. PMID: 25278026
    27. knocking out the DNA sensor cyclic GMP-AMP synthase completely abrogates spontaneous induction of IFN-stimulated genes in TREX1-deficient cells. PMID: 24813208
    28. Data indicate that the single nucleotide polymorphism rs3135945 was significantly associated with HIV infection, emphasizing the involvement of TREX-1 in the anti-HIV response. PMID: 23773365
    29. This study contributes to the demonstration that TREX1 is involved in autoimmune diseases and proposes that the spectrum of involved autoimmune diseases can be broader and includes systemic sclerosis . PMID: 24224166
    30. Spontaneous type I INF dependent cutaneous pathology in TREX1 deficiency illustrates common pathogenetic pathway in chilblain lupus. PMID: 24270665
    31. These data provide compelling evidence for the required TREX1 dimeric structure for full catalytic function. PMID: 24616097
    32. A nationwide survey of Aicardi-Goutieres syndrome patients identifies a strong association between dominant TREX1 mutations and chilblain lesions in Japan. PMID: 24300241
    33. Screening of our patient cohort yielded heterozygous TREX1 mutations in two patients with early-onset cerebrovascular disease PMID: 23881107
    34. TREX1 mutation is not a common cause of hereditary small vessel diseases of the brain PMID: 23602593
    35. TREX1 post-translational modification indicates an additional mechanism by which mutations disrupt TREX1 biology, leading to human autoimmune disease. PMID: 23979357
    36. TREX1 gene variants confer an increased risk for the development of systemic lupus erythematosus. PMID: 24034389
    37. TREX1 knockdown resulted in enhanced cell death following nimustine. PMID: 23578789
    38. Aicardi-Goutieres syndrome and familial chilblain lupus can be caused by a heterozygous TREX1 p.Asp18Asn mutation. PMID: 22829693
    39. Despite its central role in the HIV-1 infection process, genetic diversity at TREX1 is not a major determinant of susceptibility to infection in humans. PMID: 22526516
    40. The structures of the mutant TREX1 proteins provide insight into the dysfunction relating to human disease. PMID: 22071149
    41. TREX1 residues in one protomer contributing to DNA degradation catalyzed in the opposing protomer and help to explain the dimeric TREX1 structure required for full catalytic competency. PMID: 21937424
    42. failure to degrade genomic dsDNA is a principal pathway of immune activation in TREX1-mediated autoimmune disease. PMID: 21808053
    43. data confirm and expand previous reports and provide additional support for the involvement of TREX1 in lupus pathogenesis PMID: 21270825
    44. A single case of a heterozygous TREX1 mutation has been associated with Aicardi-Goutieres syndrome, an encephalopathy often resembling congenital infection. PMID: 20799324
    45. TREX1 bound to cytosolic HIV DNA and digested excess HIV DNA that would otherwise activate interferon expression via a pathway dependent on the kinase TBK1, the adaptor STING and the transcription factor IRF3 PMID: 20871604
    46. The deficiency of Trex-1 in rheumatoid arthritis synovial fibroblasts allows a longer half-life of gene products encoded by active endogenous L1 retrotransposons. PMID: 20496420
    47. TREX1 mutations do not play a role in the pathogenesis of Sporadic inclusion body myositis. PMID: 20192983
    48. TREX1 may act in degrading DNA in all cell types undergoing a dying process before phagocytosis occurs. PMID: 19617005
    49. TREX1, encoding the major mammalian 3' --> 5' DNA exonuclease, is the AGS1 gene, and AGS-causing mutations result in abrogation of TREX1 enzyme activity. PMID: 16845398
    50. Heterozygous mutations in TREX1 cause familial chilblain lupus and dominant Aicardi-Goutieres syndrome. PMID: 17357087

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  • 相关疾病：
    Aicardi-Goutieres syndrome 1 (AGS1); Systemic lupus erythematosus (SLE); Chilblain lupus 1 (CHBL1); Vasculopathy, retinal, with cerebral leukodystrophy (RVCL)
  • 亚细胞定位：
    Nucleus. Cytoplasm, cytosol. Endoplasmic reticulum membrane; Peripheral membrane protein.
  • 蛋白家族：
    Exonuclease superfamily, TREX family
  • 组织特异性：
    Detected in thymus, spleen, liver, brain, heart, small intestine and colon.
  • 数据库链接：

    HGNC: 12269

    OMIM: 152700

    KEGG: hsa:11277

    STRING: 9606.ENSP00000390478

    UniGene: Hs.707026